Ben-Ami H, Edoute Y
Medical Dept. C, Rambam Medical Center, Haifa.
Harefuah. 1998 Feb 1;134(3):178-80, 247.
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired disorder of blood cells which originate from an abnormal hematopoietic stem cell. The condition is characterized by nocturnal hemoglobinuria, chronic hemolytic anemia, and thrombosis. We describe a 60-year-old woman with PNH admitted with abdominal pain and jaundice, who had dark urine on arising after a night's sleep. The diagnosis was established by the typical clinical story and a positive Ham test. She was successfully treated with Halotestin and folic acid. Although PNH is rare, it should be considered in the differential diagnosis of hemolytic anemia. Early diagnosis and treatment are important.
阵发性夜间血红蛋白尿(PNH)是一种罕见的后天性血细胞疾病,起源于异常造血干细胞。该病症的特征为夜间血红蛋白尿、慢性溶血性贫血和血栓形成。我们描述了一名60岁患有PNH的女性,因腹痛和黄疸入院,她在睡了一夜后起床时尿液呈深色。通过典型的临床症状和阳性的酸溶血试验确诊。她接受氟羟甲睾酮和叶酸治疗后康复。尽管PNH罕见,但在溶血性贫血的鉴别诊断中应予以考虑。早期诊断和治疗很重要。